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T2 - Phase 2 - Systems Modules (II) > Renal - Neoplasia > Flashcards

Renal - Neoplasia Flashcards

1
Q

What is an angiomyolipoma?

A

A hamartoma

(comprised of blood vessels, smooth muscle. and adipose)

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2
Q

Angiomyolipomas are hamartomas associated with what condition?

A

Tuberous sclerosis

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3
Q

Angiomyolipomas are hamartomas comprised of what tissue type(s)?

A

Blood vessels;

smooth muscle;

adipose tissue

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4
Q

Angiomyolipomas are benign renal neoplasms found primarily in what organ(s)?

A

The kidneys

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5
Q

Renal cell carcinoma is a malignant epithelial tumor arising from what?

A

The kidney tubules

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6
Q

What is the classic triad of renal cell carcinoma presentation?

A

(1) Hematuria
(2) Palpable mass
(3) Flank pain

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7
Q

The classic triad of renal cell carcinoma presentation is hematuria, palpable mass, and flank pain. How often do they occur simultaneously?

A

Very rarely

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8
Q

What is the most common presenting symptom of a renal cell carcinoma?

A

Hematuria

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9
Q

Name a few paraneoplastic syndromes associated with renal cell carcinomas.

A

EPO secretion

Renin secretion

PTH-related-peptide secretion

ACTH secretion

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10
Q

True/False.

Commonly, renal cell carcinomas present as left-sided varicocele.

A

False.

Rarely, renal cell carcinomas present as left-sided varicocele.

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11
Q

Gross exam of a renal cell carcinoma reveals what?

A

A yellow mass

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12
Q

Microscopically, the most common subtype of renal cell carcinoma presents how?

A

Clear cytoplasm

(clear cell subtype)

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13
Q

Pathogenesis of renal cell carcinomas involves a loss of ______ (tumor suppressor), which leads to increased ______ (growth factor) and ______ (growth factor).

A

Pathogenesis of renal cell carcinomas involves a loss of VHL, which leads to increased IGF-1 and HIF-1-α.

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14
Q

HIF-1α increases secretion of which transcription factors?

A

VEGF and PDGF

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15
Q

Are renal cell carcinomas hereditary or sporadic?

A

Can be either!

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16
Q

Hereditary renal cell carcinomas are most commonly seen in what condition?

A

von Hippel-Lindau disease

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17
Q

von Hippel-Lindau disease typically presents in ______ (age group) and 50% present with ______ (laterality) renal cell carcinomas.

A

von Hippel-Lindau disease typically presents in young adults and 50% present with bilateral renal cell carcinomas.

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18
Q

Sporadic renal cell carcinomas classically arise in _________ (patient population) as a single tumor in the upper pole of the kidney.

A

Sporadic renal cell carcinomas classically arise in adult males as a single tumor in the upper pole of the kidney.

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19
Q

Sporadic renal cell carcinomas classically arise in adult males as a single tumor in what location?

A

The upper pole of the kidney

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20
Q

What is the major risk factor for sporadic renal cell carcinomas?

A

Cigarette smoke

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21
Q

Renal cell carcinoma TNM staging is unique in that the tumor analysis involves assessment of the tumor’s interaction with what?

A

The renal vein

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22
Q

In addition to their spread to the renal vein, renal cell carcinomas commonly spread to which lymph nodes?

A

The retroperitoneal lymph nodes

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23
Q

Wilms tumor is a malignant tumor of the ________ that is comprised of immature mesenchyme known as ________.

A

Wilms tumor is a malignant tumor of the kidney that is comprised of immature mesenchyme known as blastema.

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24
Q

True/False.

Wilms tumors are benign tumors comprised of blastema (mature renal mesenchyme), primitive glomeruli/tubules, and some stromal cells.

A

False.

Wilms tumors are malignant tumors comprised of blastema (immature renal mesenchyme), primitive glomeruli/tubules, and some stromal cells.

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25
Q

A child presents with a large, unilateral flank mass. What is the most common renal tumor in children?

A

Wilms tumor

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26
Q

Describe the clinical presentation associated with Wilms tumor.

A

A child with a large, unilateral flank mass, hypertension, and hematuria.

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27
Q

Why do children with Wilms tumor present with hypertension?

A

Excess renin secretion by the tumor

28
Q

Most cases (90%) of Wilms tumors are ___________ (etiology).

A

Most cases (90%) of Wilms tumors are sporadic.

29
Q

Although most cases of WIlms tumor are sporadic, Wilms tumors are also associated with some syndromes. Name three.

A

WAGR syndrome

Denys-Drash syndrome

Beckwith-Wiedemann syndrome

30
Q

Describe the features of WAGR syndrome.

A

Wilms tumors

Aniridia

Genital abnormalities

Motor Retardation

31
Q

WAGR syndrome is associated with deletions in what gene?

A

WT1

32
Q

Describe the features of Denys-Drash syndrome.

A

(1) Wilms tumor
(2) Progressive renal disease
(3) Male pseudohermaphroditism

33
Q

Denys-Drash syndrome is associated with mutations in what gene?

A

WT1

34
Q

Describe the features of Beckwith-Wiedemann syndrome.

A

(1) Wilms tumor
(2) Neonatal hypoglycemia
(3) Muscular hemihypertrophy
(4) Organomegaly

35
Q

Beckwith-Wiedemann syndrome is associated with mutations in what gene?

A

WT2 cluster

(particularly IGF-2)

36
Q

What disorder is described below?

A 3-year-old child presents with aniridia, a large, left flank mass that does not cross the midline, genital abnormalities, and some lack of motor function.

A

WAGR syndrome

(Wilms tumors

Aniridia

Genital abnormalities

Motor Retardation)

37
Q

What disorder is described below?

A 2-year-old child presents with a large, left flank mass that does not cross the midline, ambiguous external genitalia, and elevated serum BUN. Karyotyping reveals that the child is XY.

A

Denys-Drash syndrome

  • ((1) Wilms tumor*
  • (2) Progressive renal disease*
  • (3) Male pseudohermaphroditism)*
38
Q

What disorder is described below?

A newborn presents with umbilical hernia, hypoglycemia, overgrowth of the lower extremity musculature, and macrosomia. Two years later, the patient is found to have a large, unilateral right flank mass that does not cross the midline.

A

Beckwith-Wiedemann syndrome

  • ((1) Wilms tumor*
  • (2) Neonatal hypoglycemia*
  • (3) Muscular hemihypertrophy*
  • (4) Organomegaly)*
39
Q

Malignancies of the lower urinary tract are defined as malignancies arising in what locations?

A

Renal pelvis;

ureter;

bladder;

urethra

40
Q

Most malignancies of the lower urinary tract (squamous cell, urothelial, adenocarcinoma) occur in what organ?

A

The bladder

41
Q

What is the most common type of malignancy of the lower urinary tract?

A

Urothelial carcinoma

(a.k.a. transitional cell carcinoma)

42
Q

Urothelial carcinomas (transitional cell carcinomas) most commonly arise in what location?

A

The bladder

43
Q

Urothelial carcinomas (transitional cell carcinomas) are most commonly seen in what patient population?

A

Older adults

44
Q

What is the major presenting sign/symptom of urothelial (transitional cell) carcinomas?

A

Painless hematuria

45
Q

What is the major risk factor for urothelial (transitional cell) carcinomas?

Can you name any others?

A

Cigarette smoke;

naphthylamine, azo dyes, long-term cyclophosphamide or phenacetin use

46
Q

Urothelial (transitional cell) carcinomas are often ______focal and _______ (do/do not) recur.

A

Urothelial (transitional cell) carcinomas are often multifocal and do recur.

47
Q

What are the two main pathways from which urothelial (transitional cell) carcinomas arise?

A

(1) Flat (early P53 mutations)
(2) Papillary

48
Q

Which pathway of urothelial (transitional cell) carcinoma is associated with early P53 mutations?

A

The flat pathway

(as opposed to papillary)

49
Q

Describe the progression of the flat pathway of urothelial (transitional cell) carcinomas.

A

Develops as a high-grade, flat tumor; then invades

50
Q

Describe the progression of the papillary pathway of urothelial (transitional cell) carcinomas.

A

Develops as a low-grade, papillary tumor; then progresses to a high-grade tumor; then invades

51
Q

Which form of urothelial (transitional cell) carcinoma begins as a high-grade tumor, invading soon after?

A

The flat form

52
Q

Which form of urothelial (transitional cell) carcinoma begins as a low-grade tumor and then progresses to a high-grade tumor before invading?

A

The papillary form

53
Q

Which form of urothelial (transitional cell) carcinoma is associated with early P53 mutations?

A

The flat form

54
Q

True/False.

Urothelial (transitional cell) carcinomas are often multifocal, but they typically respond well to typical resection.

A

False.

Urothelial (transitional cell) carcinomas are often multifocal, and they recur.

55
Q

Squamous cell carcinomas of the lower urinary tract typically involve which organ?

A

The bladder

56
Q

What typically must occur before a squamous cell carcinoma can arise in the lower urinary tract?

A

Squamous metaplasia

(normal bladder and urinary tract surface is lined by urothelium)

57
Q

Schistosoma haematobium is associated with what malignancy?

A

Squamous cell carcinoma of the bladder

58
Q

Squamous cell carcinomas of the bladder associated with Schistosoma haematobium are most commonly seen in what patient population?

A

Egyptian males

59
Q

Squamous cell carcinoma of the bladder that arises in an older woman is associated with what risk factor?

A

Chronic cystitis

60
Q

True/False.

The major risk factor for squamous cell carcinoma of the lower urinary tract is cigarette use.

A

False.

The major risk factor for urothelial (transitional cell) carcinoma of the lower urinary tract is cigarette use.

61
Q

Long-standing nephrolithiasis is a risk factor for what malignancy of the lower urinary tract?

A

Squamous cell carcinoma

62
Q

___________ of the lower urinary tract is a malignant proliferation of glandular tissue, most often arising in the bladder.

A

Adenocarcinoma of the lower urinary tract is a malignant proliferation of glandular tissue, most often arising in the bladder.

63
Q

A glandular malignancy arising at the dome of the bladder is most likely to be arising from what?

A

The urachal remnant

(The malignancy is an adenocarcinoma.)

64
Q

Name an inflammatory condition that increases the risk of adenocarcinoma of the bladder.

A

Cystitis glandularis

65
Q

Name a congenital abnormality that increases the risk of adenocarcinoma of the bladder.

A

Extrophy

66
Q

Describe the histology of clear cell renal cell carcinomas.

A

Clear cells (in an acinar growth pattern) with intricate “chickenwire” vasculature

T2 - Phase 2 - Systems Modules (II) (22 decks)

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